title: Extradural hematoma surgery in a child with Hutchinson-Gilford progeria syndrome: Perioperative concerns.
abstract: Hutchinson-Gilford progeria syndrome (HGPS) is a very rare genetic disorder characterized by premature ageing, severe growth failure, and very early onset atherosclerosis. Psychologically and emotionally child-like, these patients suffer from physiological changes of old age. Early and progressive atherosclerosis of intra-cranial vessels in HGPS patients, along with a thin skin and fragile vessels, make these patients susceptible to intra-cranial hematomas following relatively trivial injuries and to severe intra-cranial disease. Anesthetizing HGPS patients for surgery can be challenging due to the presence of a possible difficult airway, multi-system derangements, and associated skin, bone and joint disease. We report here one such child with HGPS who underwent craniotomy and evacuation of an extradural hematoma that developed after minor head trauma. Securing his airway during surgery was difficult.
authors.affiliation: Department of Anaesthesiology, SCB Medical College, Cuttack, India ; Department of Anaesthesiology Acharya Harihar Regional Cancer Centre, Cuttack, India.
authors.forename: Upendra
authors.initials: U
authors.lastname: Hansda
authors.forename: Jyotsna
authors.initials: J
authors.lastname: Agarwal
authors.forename: Chitralekha
authors.initials: C
authors.lastname: Patra
authors.forename: Pragati
authors.initials: P
authors.lastname: Ganjoo
keywords: Anesthesia; Hutchinson–Gilford progeria syndrome; atherosclerosis; intra-cranial hematoma; neurosurgery; progeria